Manifestations and treatment of congenital fibrinogen deficiency

Ruchika Sharma, Bryce A. Kerlin

Research output: Chapter in Book/Report/Conference proceedingChapter


Fibrinogen is a complex glycoprotein that polymerizes following cleavage by thrombin to form the fibrin clot. Congenital fibrinogen disorders are heterogeneous and comprise qualitative and quantitative defects in fibrinogen. These may present as bleeding or thrombosis phenotypes. Congenital fibrinogen disorders result from mutations in any of the three genes (FGA, FGB, and FGG), and both autosomal dominant and recessive forms exist. Replacement therapy with fibrinogen concentrate may be warranted in some patients.

Original languageEnglish (US)
Title of host publicationPediatric Bleeding Disorders
Subtitle of host publicationA Clinical Casebook
PublisherSpringer International Publishing
Number of pages8
ISBN (Electronic)9783030316617
ISBN (Print)9783030316600
StatePublished - Jan 1 2020
Externally publishedYes


  • Afibrinogenemia
  • Bleeding or thrombosis
  • Dysfibrinogenemia
  • Fibrinogen
  • Fibrinogen concentrate
  • Hypofibrinogenemia

ASJC Scopus subject areas

  • Medicine(all)


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